Energy metabolism in ALS: an underappreciated opportunity?
- Vandoorne T Department of Neurosciences, Experimental Neurology, KU Leuven-University of Leuven, Campus Gasthuisberg O&N 4, Herestraat 49, PB 602, 3000, Leuven, Belgium.
- De Bock K Laboratory of Exercise and Health, Department of Health Sciences and Technology, ETH Zurich, Zurich, Switzerland.
- Van Den Bosch L Department of Neurosciences, Experimental Neurology, KU Leuven-University of Leuven, Campus Gasthuisberg O&N 4, Herestraat 49, PB 602, 3000, Leuven, Belgium. ludo.vandenbosch@kuleuven.vib.be.
- 2018-03-18
Published in:
- Acta neuropathologica. - 2018
Amyotrophic lateral sclerosis
Energy metabolism
Metabolic dysfunction
Metabolic treatment
Mitochondria
Neuron-glia metabolic coupling
Amyotrophic Lateral Sclerosis
Animals
Energy Metabolism
Humans
English
Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive and fatal neurodegenerative disorder that primarily affects motor neurons. Despite our increased understanding of the genetic factors contributing to ALS, no effective treatment is available. A growing body of evidence shows disturbances in energy metabolism in ALS. Moreover, the remarkable vulnerability of motor neurons to ATP depletion has become increasingly clear. Here, we review metabolic alterations present in ALS patients and models, discuss the selective vulnerability of motor neurons to energetic stress, and provide an overview of tested and emerging metabolic approaches to treat ALS. We believe that a further understanding of the metabolic biology of ALS can lead to the identification of novel therapeutic targets.
- Language
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- English
- Open access status
- hybrid
- Identifiers
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- DOI 10.1007/s00401-018-1835-x
- PMID 29549424
- Persistent URL
- https://folia.unifr.ch/global/documents/267575
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